PB2265: RISK FACTORS FOR DISEASE PROGRESSION OF MYCOSIS FUNGOIDES AND SÉZARY SYNDROME IN EAST ASIAN POPULATION
Authors: Huai-Chueh Gem Wu, Hsin-Yu Huang, Yenlin Huang, Kung-Chao Chang, Cheng-Lin Wu
Journal: HemaSphere (2023)

Topic: 18. Indolent and mantle-cell non-Hodgkin lymphoma - Clinical Background: The Cutaneous Lymphoma International Prognostic Index (CLIPi) was developed by Benton et al. in 2013 for the risk stratification of mycosis fungoides (MF) and Sezary syndrome (SS). While the CLIPi has been shown to be a useful prognostic tool for patients with MF/SS. It is unclear whether the CLIPi can be validated in an East Asian-based cohort and whether modifications are necessary to optimize its predictive value. Moreover, the primary outcome of the CLIPi was survival, and there is limited knowledge of the impact of some clinical and pathological risk factors on its performance. Aims: This study aims to evaluate the applicability of the CLIPi in an East Asian-based cohort of patients with MF and SS. Specifically, we aim to validate the performance of the CLIPi in this cohort, identify potential risk factors that may modify the index’s predictive value, and investigate the impact of clinical and pathological factors, beyond those used in the original CLIPi, on disease progression and recurrence in patients with MF/SS. Methods: We retrospectively reviewed the medical records and histopathological findings of adult patients with MF or SS who were diagnosed and treated at one tertiary center in Taiwan between 2008-2022. We collected clinical and pathological data, including age, sex, comorbidities, previous dermatological disease, morphological characteristics of MF lesion (patch/plaque/tumor/erythroderma), lesion distribution (head and neck, trunk, extremities), lymphadenopathies, TNMB stage, biochemistry tests [lactate dehydrogenase and albumin levels], blood cell counts (leukocytes, platelets, eosinophils, lymphocytes, atypical lymphocytes), and treatment (phototherapy with ultraviolet, radiotherapy, chemotherapy). Eosinophil infiltration counts and immunohistochemical staining for CD2, CD3, CD4, CD5, CD7, CD8, and CD30 were performed. The primary outcomes were all-cause mortality and recurrence. Patients were followed up until Feb. 2023, or death, whichever occurred first. We assessed the association between clinical variables and outcomes using univariate and multivariate analyses and Cox regression models. Statistical analysis was performed using SPSS IBM 25. A p-value less than 0.05 was considered statistically significant. The study was approved by the institutional review board (B-ER-101-042). Results: We included 102 patients, and the median age of the population was 51 years old, and 56.8% (n=58) were male. The stage distribution was as follows: IA (n=51), IB (n=30), II (n=12), III (n=6), IV (n=3). The overall survival rate was 87.3%, and the median overall survival was 68 months. The rate of disease progression was 59.8%, and the median progression-free survival was 36 months. Univariate analysis revealed several clinical factors associated with an increased risk of disease progression, including non-patch presentation (hazard ratio, HR 1.87, p = 0.035), tumor lesion (HR 2.46, p = 0.010), erythroderma (HR 3.75, p = 0.001), presence of B symptoms (HR 5.38, p < 0.001), elevated LDH (HR 2.815, p = 0.018), and thrombocytopenia (HR 3.345, p = 0.013). We are currently reviewing the histopathological features of the tumor specimens to identify additional risk factors associated with disease progression and recurrence. Summary/Conclusion: The identification of these factors may aid in risk stratification and treatment decision-making for patients with MF/SS. Further investigation of the histopathological features may provide additional insight into the pathogenesis of these diseases and inform the development of more effective treatmentsKeywords: Mycosis fungoides, Cutaneous T-cell lymphoma, cutaneous lymphoma, Progression